title: [Eculizumab for the treatment of atypical hemolytic uremic syndrome: case report and revision of the literature].
abstract: SHU atypical (aHUS), that is, not associated with Escherichia coli Shiga toxinproducing, is seen in 5 to 10% of cases of Hemolytic Uremic Syndrome (HUS), and can occur at any age and may be sporadic or familial. The prognosis in these cases is reserved, with high mortality and morbidity in the acute phase of the disease, and about 50% of cases can develop chronic kidney disease. The increased knowledge of the pathogenesis of aHUS (overactivation of the alternative pathway of complement), was accompanied by the appearance of a drug, eculizumab, which acts as an inhibitor of membrane attack complex. Our goal is to report a case of infant with aHUS with excellent clinical and laboratory response with the use of eculizumab. 14 month old infant, previously healthy, male, presented anemia and thrombocytopenia at 12 months of age. He was treated with corticosteroids and forwarded to our service for high blood pressure. However, the scans showed nephrotic proteinuria with renal involvement and hypoalbuminemia with direct Coombs negative. He developed anemia, thrombocytopenia, worsening of renal function and hypertension. Renal biopsy showed thrombotic microangiopathy (TMA). On the non-hemolytic anemia, thrombocytopenia and acute renal failure with histological substrate MAT, was diagnosed of aHUS. The patient received eculizumab excellent clinical and laboratory response. This case shows the importance of early diagnosis and treatment of the aHUS. Eculizumab is effective and keeps long-term remission, avoiding invasive measures such as plasmapheresis, which resolves only part of the picture.
authors.forename: Maria Helena
authors.initials: MH
authors.lastname: Vaisbich
authors.forename: Luciana Dos Santos
authors.initials: Ldos S
authors.lastname: Henriques
authors.forename: Andréia
authors.initials: A
authors.lastname: Watanabe
authors.forename: Lilian Monteiro
authors.initials: LM
authors.lastname: Pereira
authors.forename: Camila Cardoso
authors.initials: CC
authors.lastname: Metran
authors.forename: Denise Avancini
authors.initials: DA
authors.lastname: Malheiros
authors.forename: Flávia
authors.initials: F
authors.lastname: Modanez
authors.forename: João Domingos Montoni da
authors.initials: JD
authors.lastname: Silva
authors.forename: Simone
authors.initials: S
authors.lastname: Vieira
authors.forename: Ana Catarina Lunz
authors.initials: AC
authors.lastname: Macedo
authors.forename: Bianca
authors.initials: B
authors.lastname: Massarope
authors.forename: Erika Arai
authors.initials: EA
authors.lastname: Furusawa
authors.forename: Benita Galassi Soares
authors.initials: BG
authors.lastname: Schvartsman
mesh_terms: D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use*; D065766:Atypical Hemolytic Uremic Syndrome; D006463:Hemolytic-Uremic Syndrome / Q000188:drug therapy*; D006801:Humans; D007223:Infant; D008297:Male
chemical_list: D061067:Antibodies, Monoclonal, Humanized; C481642:eculizumab